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Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH), An Under Recognized Clinical Entity
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A6736 - Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH), An Under Recognized Clinical Entity
Author Block: A. Divakaran1, S. Bhattacharya1, W. Khan2; 1Internal Medicine, Robert Wood Johnson Medical School, New Brunswick, NJ, United States, 2Pulmonary and Critical Care, University Medical Center of Princeton at Plainsboro, Plainsboro, NJ, United States.
Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a very rare proliferation of neuroendocrine cells within the lung. It is considered a pre-malignant condition. DIPNECH has been described only in case reports and small case series. The typical presentation is of a middle-aged woman with unexplained chronic cough. Radiographic imaging typically reveals small pulmonary nodules, and sometimes ground-glass lesions or air trapping. Pulmonary function testing can be normal or show an obstructive pattern. Definitive diagnosis of DIPNECH requires tissue sampling, which reveals pulmonary neuroendocrine cell islets. Due to its rarity, the condition is often misdiagnosed. We present the case of a woman presenting with unexplained chronic cough for ten years.
Case : A 52-year old woman with PMH of HTN presented to our outpatient pulmonary clinic due to chronic cough. She reported a dry cough for approximately ten years. There was no associated dyspnea or sputum production. The cough had previously been attributed to post-nasal drip or gastroesophageal reflux. She underwent a colonoscopy for routine colon cancer screening, which revealed one carcinoid polyp. She then underwent surveillance computed tomography of the chest, abdomen, and pelvis, which revealed multiple subcentimeter pulmonary nodules. There was also a dominant 1 x 1.3 cm nodule in the left upper lobe. She underwent surgical resection of the dominant nodule. Pathology revealed neuroendocrine islet cells. Treatment was initiated with sandostatin and the cough improved.
Discussion: DIPNECH is very rare and often misdiagnosed as chronic obstructive lung disease, upper airway cough syndrome, gastroesophageal reflux or other causes of cough. Misdiagnosis is concerning as it is considered a pre-malignant condition. Accordingly, in this case the diagnosis was overlooked for approximately ten years. Pathology ultimately confirmed neuroendocrine tumor islet proliferation. Treatment for DIPNECH usually includes sandostatin. Oral glucocorticoids and inhaled bronchodilators have been used to manage cough with varying degrees of success. Prognosis is difficult to assess due to limited cases, however appears to be good. Most cases demonstrate clinical improvement or stabilization.
Author Block: A. Divakaran1, S. Bhattacharya1, W. Khan2; 1Internal Medicine, Robert Wood Johnson Medical School, New Brunswick, NJ, United States, 2Pulmonary and Critical Care, University Medical Center of Princeton at Plainsboro, Plainsboro, NJ, United States.
Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a very rare proliferation of neuroendocrine cells within the lung. It is considered a pre-malignant condition. DIPNECH has been described only in case reports and small case series. The typical presentation is of a middle-aged woman with unexplained chronic cough. Radiographic imaging typically reveals small pulmonary nodules, and sometimes ground-glass lesions or air trapping. Pulmonary function testing can be normal or show an obstructive pattern. Definitive diagnosis of DIPNECH requires tissue sampling, which reveals pulmonary neuroendocrine cell islets. Due to its rarity, the condition is often misdiagnosed. We present the case of a woman presenting with unexplained chronic cough for ten years.
Case : A 52-year old woman with PMH of HTN presented to our outpatient pulmonary clinic due to chronic cough. She reported a dry cough for approximately ten years. There was no associated dyspnea or sputum production. The cough had previously been attributed to post-nasal drip or gastroesophageal reflux. She underwent a colonoscopy for routine colon cancer screening, which revealed one carcinoid polyp. She then underwent surveillance computed tomography of the chest, abdomen, and pelvis, which revealed multiple subcentimeter pulmonary nodules. There was also a dominant 1 x 1.3 cm nodule in the left upper lobe. She underwent surgical resection of the dominant nodule. Pathology revealed neuroendocrine islet cells. Treatment was initiated with sandostatin and the cough improved.
Discussion: DIPNECH is very rare and often misdiagnosed as chronic obstructive lung disease, upper airway cough syndrome, gastroesophageal reflux or other causes of cough. Misdiagnosis is concerning as it is considered a pre-malignant condition. Accordingly, in this case the diagnosis was overlooked for approximately ten years. Pathology ultimately confirmed neuroendocrine tumor islet proliferation. Treatment for DIPNECH usually includes sandostatin. Oral glucocorticoids and inhaled bronchodilators have been used to manage cough with varying degrees of success. Prognosis is difficult to assess due to limited cases, however appears to be good. Most cases demonstrate clinical improvement or stabilization.
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